Secondary mitochondrial dysfunction in propionic aciduria: A pathogenic role for endogenous mitochondrial toxins

Biochemical Journal

Volumn 398, Issue 1, 2006, Pages 107-112

  Schwab, Marina A ^a   Sauer, Sven W ^a   Okun, Jürgen G ^a   Nijtmans, Leo G J ^b   Rodenburg, Richard J T ^b   Van Den Heuvel, Lambert P ^b   Dröse, Stefan ^c   Brandt, Ulrich ^c   Hoffmann, Georg F ^a   Ter Laak, Henk ^b   Kölker, Stefan ^a   Smeitink, Jan A M ^b  

^a UNIVERSITY HOSPITAL HEIDELBERG   (Germany)

^b RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

^c GOETHE UNIVERSITY   (Germany)

Author keywords

Mitochondrial dysfunction; Mitochondrial toxin; Oxidative phosphorylation; Propionic aciduria; Propionyl CoA; Pyruvate dehydrogenase complex

Indexed keywords

COMPLEXATION; CYTOLOGY; ENZYMES; FATTY ACIDS; TOXIC MATERIALS;

MITOCHONDRIAL DYSFUNCTION; MITOCHONDRIAL TOXIN; OXIDATIVE PHOSPHORYLATION; PROPIONIC ACIDURIA; PROPIONYL-COA; PYRUVATE DEHYDROGENASE COMPLEX;

PATHOLOGY;

ACETYL COENZYME A; ACYL COENZYME A; CYTOCHROME C OXIDASE; MITOCHONDRIAL DNA; OXOGLUTARATE DEHYDROGENASE; PROPIONYL COENZYME A; PYRUVATE DEHYDROGENASE COMPLEX; RESPIRATORY COMPLEX I; RESPIRATORY COMPLEX II; RESPIRATORY COMPLEX III; TRICARBOXYLIC ACID; UNCLASSIFIED DRUG;

ACIDURIA; ANAMNESIS; ANIMAL TISSUE; ARTICLE; BIOENERGY; CASE REPORT; CLINICAL FEATURE; CONTROLLED STUDY; DISORDERS OF MITOCHONDRIAL FUNCTIONS; ENERGY METABOLISM; ENZYME ACTIVITY; FEMALE; HUMAN; HUMAN TISSUE; IN VITRO STUDY; INHIBITION KINETICS; MALE; MUSCLE BIOPSY; NONHUMAN; OXIDATIVE PHOSPHORYLATION; PATHOGENESIS; PATHOGENICITY; PRIORITY JOURNAL; PROTEIN EXPRESSION; RESPIRATORY CHAIN;

ACETYL COENZYME A; ACYL COENZYME A; AMINO ACID METABOLISM, INBORN ERRORS; ANIMALS; CATTLE; ENERGY METABOLISM; FATTY ACIDS; FEMALE; FIBROBLASTS; HUMANS; INFANT, NEWBORN; MALE; MITOCHONDRIAL DISEASES; OXIDATIVE PHOSPHORYLATION; PROPIONIC ACIDS; PYRUVATE DEHYDROGENASE COMPLEX; QUADRICEPS MUSCLE; SKIN; SWINE; TOXINS, BIOLOGICAL;

BOS TAURUS; SUIDAE;

EID: 33747167133     PISSN: 02646021     EISSN: None     Source Type: Journal    
DOI: 10.1042/BJ20060221     Document Type: Article

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